CASE STUDY A 51-YEAR-OLD WOMAN WITH END-STAGE COPD — Gerard J. Criner, MD BACKGROUND A 51-year-old African-American female patient presented to the health center’s emergency department with severe dyspnea on exertion and progressive edema of the lower extremities. She reported that her breathlessness and coughing episodes had become particularly severe in the preceding 3 weeks and was now at the point where she could not stand or walk for more than 1 minute to 2 minutes without becoming fatigued. HISTORY She is a 40 pack-year smoker (ie, about 1 and 1/2 packs per day since her mid-20s) and currently still smokes cigarettes. She is not taking any medications and has no family history of asthma, allergy, or cardiovascular disease. She claims to have never been treated by a doctor. PHYSICAL EXAMINATION Woman with moderately above normal body mass index who is in obvious respiratory distress. Decreased breath sounds and obvious cyanotic skin. 2+ lower extremity edema to thigh. She had neck vein distention and a positive hepatojugular reflex. S420 Respiratory rate: Heart rate: SaO2: 30 per minute 115 bpm 78% Arterial blood gases: PaO2: PaCO2: pH: HCT: Prealbumin: 41 mm Hg 66 mm Hg 7.28 48% low normal Repeat arterial blood gases: 113 mm Hg PaO2: 92 mm Hg PaCO2: pH: 7.28 FURTHER EVALUATIONS Echocardiogram revealed severe right ventricular hypokinesis and a right ventricular systolic pressure of 55 mm Hg. Catheterization of the right side of the heart showed a pressure of 70/30 (48) mm Hg and a cardiac output of 4 L/minutes with no O2 step-up. The forced expiratory volume in 1 second (FEV1) was 0.38 L (16%); the residual volume was 5.97 L (373%); and the ratio of residual volume to total lung capacity was 84%. Diffusion capacity was 7.8 mL/mm Hg/min (41%). The ventilation/perfusion matched abnormality in both lower lobes, and there was 7% quantitative perfusion to the bases, demonstrating oligemia to the lower lobes. MANAGEMENT The patient was deemed to be in acute respiratory distress and to have severe smoking-related chronic Vol. 3 (5B) ■ May 2003 CASE STUDY obstructive pulmonary disease (COPD). She was gas smoking-cessation program, earlier introduction of trapped and hyperinflated without significant diffuse medical therapy might have delayed and possibly emphysema. After immediate admission to the intenaverted the need for transplantation in this patient. sive care unit, she was started on bronchodilators, sysThe complete absence of medical evaluation, counseltemic steroids, inhaled steroids, antibiotics, and ing, or therapy for this lifelong smoker is, unfortunoninvasive positive pressure ventilation (NPPV). nately, common. In this worst-case scenario, After 3 weeks of inpatient hospitalization she was disinterventions such as steroids, oxygen, and NPPV charged to her home with chronic nocturnal NPPV allowed the patient to be more comfortable while waitand supplemental oxygen. She was also referred withing for the appropriate lung procedure. Reviewing this in the health system for immediate outpatient rehabilcase of a patient with end-stage COPD should remind itation, smoking cessation, and weight monitoring. A clinicians of the reasons for instituting early and flutter valve device was provided for management of aggressive smoking-cessation efforts and the need to coughing exacerbations. develop newer medical therapies and surgical techDue to the severity of lung function deterioration niques that may prevent or at least delay the need for (eg, FEV1 well below 25%) and the inability of medthe most radical COPD solution. The demand for ical therapy to relieve symptoms, this patient was listsuch symptom-based interim therapies will increase in ed for lung transplantation. She had no significant coming years as United States health systems are change in her condition in 3 years and underwent forced to deal with that large population of patients double-lung transplantation. Performance as measured with COPD who have already progressed to the more by lung function (Figure) and by physical function advanced stages of the disease. and her quality of life have improved. As shown on the pretransplantation and post-transplantation spirometry results, she had a 350% increase in spirometric volume; she also had a reduction in Figure. Spirometry Before and After Lung Transplantation dyspnea and a marked improvement in quality of life. DISCUSSION Lung transplantation is a lifeextending extreme measure for patients with the most severe cases of COPD. It is unknown whether this complex and risky procedure could have been avoided or at least delayed with earlier intervention in this individual patient. Smoking cessation is the 1 intervention proven to alter the long-term decline of lung function in smokers. In conjunction with an aggressive Advanced Studies in Medicine ■ S421 NOTES S422 Vol. 3 (5B) ■ May 2003